ABSTRACT
OBJECTIVES: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. METHODS: We conducted a multicentre, international, retrospective cohort study. RESULTS: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. CONCLUSIONS: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.
Subject(s)
Dermatomyositis , Lung Diseases, Interstitial , Autoantibodies , Dermatomyositis/complications , Female , Humans , Interferon-Induced Helicase, IFIH1 , Lung Diseases, Interstitial/drug therapy , Middle Aged , Prognosis , Retrospective StudiesABSTRACT
La vasculitis IgA es una vasculitis de pequeño vaso mediada por inmunocomplejos. Clínicamente se caracteriza por la púrpura palpable en miembros inferiores, la afectación articular en forma de artralgias o artritis y la afectación gastrointestinal y renal (esta última marcará el mal pronóstico en adultos). Es frecuente encontrar procesos infecciosos como desencadenantes, principalmente de vías respiratorias altas. Por otro lado, el VIH causa una disfunción inmunitaria que desencadena una hipergammaglobulinemia y puede desencadenar alteraciones autoinmunes. En ocasiones este efecto se realiza sobre el endotelio vascular dando lugar a cuadros vasculíticos, aunque como forma de inicio los casos descritos en la literatura son escasos
IgA vasculitis is a small-vessel vasculitis mediated by immune complexes. In clinical terms, it is characterized by palpable purpura in the lower limbs, joint involvement in the form of arthralgia or arthritis, and gastrointestinal and renal involvement (this will mark a poorer prognosis in adults). Infectious processes, mainly in the upper respiratory tract, are frequently found to be triggers. On the other hand, human immunodeficiency virus (HIV) causes immune dysfunction, which triggers hypergammaglobulinemia and can trigger autoimmune disorders. At times, this can affect the vascular endothelium, giving rise to vasculitic manifestations, although there are few reports in the literature of its role in the presentation of HIV
Subject(s)
Humans , Female , Adult , HIV Infections/complications , HIV Infections/diagnosis , Vasculitis/etiology , Vasculitis/diagnosis , Immunoglobulin A/bloodABSTRACT
No disponible
Subject(s)
Humans , Male , Child, Preschool , Arthritis, Infectious/complications , Pyomyositis/complications , Arthritis, Infectious/diagnosis , Arthritis, Infectious/drug therapy , Radiography, Thoracic , Synovitis/diagnostic imaging , Synovitis/drug therapy , Knee/diagnostic imaging , Cloxacillin/therapeutic use , Cefotaxime/therapeutic useSubject(s)
Arthritis, Infectious/etiology , Knee Joint , Pyomyositis/complications , Child, Preschool , Humans , Male , Pyomyositis/diagnosisABSTRACT
IgA vasculitis is a small-vessel vasculitis mediated by immune complexes. In clinical terms, it is characterized by palpable purpura in the lower limbs, joint involvement in the form of arthralgia or arthritis, and gastrointestinal and renal involvement (this will mark a poorer prognosis in adults). Infectious processes, mainly in the upper respiratory tract, are frequently found to be triggers. On the other hand, human immunodeficiency virus (HIV) causes immune dysfunction, which triggers hypergammaglobulinemia and can trigger autoimmune disorders. At times, this can affect the vascular endothelium, giving rise to vasculitic manifestations, although there are few reports in the literature of its role in the presentation of HIV.
Subject(s)
HIV Infections/complications , Vasculitis/etiology , Adult , Female , HIV Infections/diagnosis , Humans , Immunoglobulin A , Vasculitis/immunologyABSTRACT
No disponible
Subject(s)
Humans , Female , Young Adult , Vasculitis, Leukocytoclastic, Cutaneous/diagnosis , Vasculitis, Leukocytoclastic, Cutaneous/etiologySubject(s)
Exercise , Hot Temperature/adverse effects , Skin Diseases, Vascular/etiology , Vasculitis, Leukocytoclastic, Cutaneous/etiology , Diagnosis, Differential , Female , Humans , Skin Diseases, Vascular/diagnosis , Time Factors , Vasculitis, Leukocytoclastic, Cutaneous/diagnosis , Young AdultABSTRACT
No disponible
Subject(s)
Humans , Male , Adult , Hemarthrosis/etiology , Hemarthrosis , Scurvy/complications , Arthrocentesis/methods , Antibodies, Antineutrophil Cytoplasmic/analysis , Blood Coagulation Disorders/complications , Diagnosis, Differential , Knee/pathology , KneeSubject(s)
Bone Diseases/diagnosis , Bone Neoplasms/diagnosis , Sarcoidosis/diagnosis , Adult , Bone Diseases/diagnostic imaging , Bone Diseases/pathology , Bone Neoplasms/pathology , Diagnosis, Differential , Female , Humans , Ilium/diagnostic imaging , Ilium/pathology , Magnetic Resonance Imaging , Sarcoidosis/diagnostic imaging , Sarcoidosis/pathologyABSTRACT
No disponible
Subject(s)
Humans , Male , Aged , Chickenpox/drug therapy , Chickenpox Vaccine/adverse effects , Chickenpox Vaccine/isolation & purification , Methotrexate/therapeutic use , Arthritis, Rheumatoid/drug therapy , Arthritis, Rheumatoid/physiopathology , Prednisone/therapeutic use , Preventive Medicine , Adrenal Cortex Hormones/therapeutic use , Immunologic Factors/therapeutic useSubject(s)
Hemarthrosis/etiology , Scurvy/diagnosis , Adult , Animals , Ascorbic Acid/blood , Feeding Behavior , Gingivitis/etiology , Hemarthrosis/diagnostic imaging , Humans , Knee Joint/diagnostic imaging , Magnetic Resonance Imaging , Male , Malnutrition/complications , Malnutrition/psychology , Milk , Schizophrenia, Paranoid/complications , Scurvy/blood , Scurvy/complications , Scurvy/psychology , YogurtABSTRACT
Mujer de 65 años de edad con antecedentes de sarcoidosis, con afectación pulmonar y articular, que tras 5 años del diagnóstico comienza con afectación del sistema nervioso central, manifestándose como diplopía. Presenta analíticas normales. En las pruebas de imagen se identifica masa intraconal derecha dependiente del nervio óptico derecho, así como múltiple afectación adenopática. Se realizó biopsia con diagnóstico de linfoma B de células grandes, forma atípica de tumor asociado a sarcoidosis (AU)
A 65 year-old female with a history of sarcoidosis with pulmonary and joint involvement, who after 5 years of diagnosis begins with central nervous system involvement manifesting as diplopia. She presents normal analysis results. In imaging results, a mass is identified in the right intraconal space; it depends of right optic nerve, and shows multiple lymph node involvement. Biopsy was performed diagnosed with large B-cell lymphoma, an atypical form of tumor associated with sarcoidosis (AU)
Subject(s)
Humans , Female , Middle Aged , Sarcoidosis/complications , Sarcoidosis , Lymphoma/complications , Lymphoma , Diplopia/complications , Diplopia/diagnosis , Radiography, Thoracic/methods , T-Lymphocytes/pathology , T-Lymphocytes , Arthralgia/complications , Panniculitis/complications , Lymphoma, Non-Hodgkin/complications , Lymphoma, Non-Hodgkin/diagnosis , Neuroimaging/instrumentation , Neuroimaging/methods , NeuroimagingABSTRACT
A 65 year-old female with a history of sarcoidosis with pulmonary and joint involvement, who after 5 years of diagnosis begins with central nervous system involvement manifesting as diplopia. She presents normal analysis results. In imaging results, a mass is identified in the right intraconal space; it depends of right optic nerve, and shows multiple lymph node involvement. Biopsy was performed diagnosed with large B-cell lymphoma, an atypical form of tumor associated with sarcoidosis.
